The dark sides of capillary morphogenesis gene 2.

Abstract

Capillary morphogenesis gene 2 (CMG2) is a type I membrane protein involved in the homeostasis of the extracellular matrix. While it shares interesting similarities with integrins, its exact molecular role is unknown. The interest and knowledge about CMG2 largely stems from the fact that it is involved in two diseases, one infectious and one genetic. CMG2 is the main receptor of the anthrax toxin, and knocking out this gene in mice renders them insensitive to infection with Bacillus anthracis spores. On the other hand, mutations in CMG2 lead to a rare but severe autosomal recessive disorder in humans called Hyaline Fibromatosis Syndrome (HFS). We will here review what is known about the structure of CMG2 and its ability to mediate anthrax toxin entry into cell. We will then describe the limited knowledge available concerning the physiological role of CMG2. Finally, we will describe HFS and the consequences of HFS-associated mutations in CMG2 at the molecular and cellular level.

• Authors: Julie Deuquet, Ekkehart Lausch, Andrea Superti-Furga, F Gisou van der Goot
• Journal: The EMBO journal (EMBO J) Vol. 31 Issue 1 Pg. 3-13 (Jan 04 2012) ISSN: 1460-2075 [Electronic] England
• PMID: 22215446 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• ANTXR2 protein, human
• Antigens, Bacterial
• Antxr2 protein, mouse
• Bacterial Toxins
• Membrane Proteins
• Receptors, Peptide
• anthrax toxin
• anthrax toxin receptors

Topics

• Animals
• Anthrax (metabolism)
• Antigens, Bacterial (metabolism)
• Bacterial Toxins (metabolism)
• Humans
• Hyalinosis, Systemic (genetics)
• Membrane Proteins (chemistry, genetics)
• Mice
• Mutation
• Protein Conformation
• Receptors, Peptide (chemistry, genetics, metabolism)